System Involvement In Children Research Articles

Child protection system involvement in children of incarcerated mothers: A linked data study

BackgroundWomen prisoners are a growing portion of the prison population. Health and social outcomes of their children have been studied and found to be poor, but little is known about child protection outcomes. ObjectivesAscertain child protection system contact of children exposed to maternal incarceration. Participants and settingAll children born between 1985 and 2011 exposed to the incarceration of their mothers in a Western Australian correctional facility and a matched comparison group. MethodsA matched cohort study using linked administrative data on 2637 mothers entering prison between 1985 and 2015 and their 6680 children. We estimated hazard ratios (HRs) and incidence rate ratios (IRRs) of child protection service (CPS) contact post maternal incarceration (four concern levels), comparing rates for children exposed to maternal incarceration with a matched non-exposed group, adjusting for maternal and child factors. FindingsExposure to maternal incarceration increased risk of CPS contact. Unadjusted HRs exposed vs unexposed children were 7.06 (95%CI = 6.49–7.69) for substantiated child maltreatment and 12.89 (95%CI = 11.42–14.55) for out-of-home care (OOHC). Unadjusted IRRs were 6.04 (95%CI = 5.57–6.55) for number of substantiations and 12.47 (95%CI = 10.65–14.59) for number of removals to OOHC. HRs and IRRs were only slightly attenuated in adjusted models. ConclusionsMaternal incarceration is a warning flag for a child at high risk of serious child protection concerns. Family-friendly rehabilitative women's prisons, incorporating support for more nurturing mother-child relationships could provide a placed-based public health opportunity for disrupting distressing life trajectories and intergenerational pathways of disadvantage of these vulnerable children and their mothers. This population should be a priority for trauma-informed family support services.

Analysis of clinical and imaging features of 6 cases of linear scleroderma en coup de sabre with central nervous system involvement in children

Objective: To summarize the clinical and imaging features of linear scleroderma en coup de saber (LSCS) with central nervous system involvement in children. Methods: The clinical data(clinical manifestations and imaging features) of 6 children diagnosed with LSCS with central nervous system involvement who were admitted to Beijing Children's Hospital Affiliated to Capital Medical University from May 2019 to November 2021 were retrospectively analyzed. Results: The 6 patients were all female, aged 6.8 (3.3, 11.0) years at the time of diagnosis, and aged 3.0 (1.7, 4.1) years at the time of discovery of facial skin lesions. Facial skin lesions appeared before neurological symptoms in 5 cases, and neurological symptoms appeared 2 months before skin lesions in 1 case. All the patients had "sword wound" skin lesions on the forehead with alopecia. Neurological manifestations included epileptic seizures in 6 cases, focal neurological defects in 5 cases, and headaches in 2 cases. The intracranial lesions were all ipsilateral to the skin lesions. The magnetic resonance imaging (MRI) of 6 cases showed abnormal signals mainly involving white matter in 1 hemisphere, and 3 cases showed local encephalomalacia. The scattered low signal was observed in 5 cases on susceptibility weighted imaging. Localized brain parenchyma or leptomeninges enhancement was seen on Gadolinium-enhanced sequences in 5 cases. Scattered foci of calcification on the affected side were seen on cranial CT in 4 cases. Skin biopsy was performed in 2 cases. Part of the lesion of the brain was removed in 1 case, and the pathological findings suggested small vasculitis, which was consistent with skin pathological changes. All patients received symptomatic treatment with antiepileptic drugs. Oral prednisone combined with methotrexate was given in 4 cases, and 1 case was given oral prednisone only. One case was presumed to be in the resting stage of the disease due to significant cerebral atrophy in half of the brain, and only antiepileptic drugs were added. The patients were followed up for 6-36 months. The skin lesions of scleroderma and alopecia did not progress in 5 cases, and hemifacial atrophy was developed in 1 case, which was considered to be combined with Parry-Romberg syndrome. The seizures were controlled in 4 cases. One case had reduced seizure frequency but left hemiplegia. One patient still had intractable epilepsy and paroxysmal headache. Conclusions: LSCS with central nervous system involvement is more common in girls, with seizures and neurological defects as the main manifestations. Intracranial lesions are mostly ipsilateral to the skin lesions. Cerebral microbleeds, calcification, and encephalomalacia foci are common, and the pathological changes in skin and intracranial lesions are consistent with small-vessel vasculitis. Prednisone combined with methotrexate treatment has shown some efficacy, but some children remain with refractory epilepsy and neurological deficit symptoms.

Zika Virus Infection and Pathogenesis

Zika virus (ZIKV) is a flavivirus that was met with relatively little acclaim when it was discovered in 1947. Initial clinical reports of ZIKV included asymptomatic infection or mild, febrile illness; however, the view of ZIKV as an insignificant virus changed dramatically following the epidemic in the Western Hemisphere that started in 2015. This epidemic featured central nervous system involvement in children and adults, and a devastating congenital syndrome following infection of pregnant women. While the pathogenicity of ZIKV was virtually undescribed prior to this epidemic, in the past few years, numerous reports have described receptor–ligand interactions, aspects of tissue tropism, host–pathogen interactions, and diversity across viral clades. In this paper, the variety of clinical presentations and virulence determinants of ZIKV are reviewed.

Respiratory Manifestations of COVID-19 in Children: A Narrative Review

Background: Coronavirus disease 2019 (COVID‐19, the seventh coronavirus) is a new infectious disease that spread worldwide and rapidly became a pandemic. COVID‐19 is rare in children and often asymptomatic. The respiratory system is mainly involved in COVID-19 infection. Objectives: This study aimed to review respiratory manifestations in children with COVID-19. Methods: We searched for studies published from January 2020 to January 2021 in PubMed, Google Scholar, and Scopus in English. The search words were “2019-nCoV,” “novel coronavirus,” “COVID-19,” “SARS-CoV-2,” “severe acute respiratory syndrome coronavirus 2,” “children,” “child,” “infant,” “teenager,” “adolescent,” “pediatric,” and “neonate.” Results: Cough was a common symptom in our study (18.8%-100%, mean=64%). Pneumonia prevalence in children with COVID-19 is 11%-67% (mean=46%). Mild respiratory symptoms (14% - 60%) and severe respiratory distress syndromes (14%) were reported in some studies. Upper respiratory tract infection (URTI) was reported in 4 studies ranging from 11.1% to 34.4% (mean=24.1%). Ground-glass opacities (GGO) was reported in 12.2% to 81% of cases (mean=49.01%). Isolated consolidations were reported in 17% and 58% of patients in two studies. Enhanced lung texture was reported in 1.3% to 50% (mean=20.07%) of patients. Bilateral patchy shadowing was seen in 12.3% to 68% (mean=46.58%) of patients. Conclusions: Cough is the most common manifestation of respiratory system involvement in children with COVID-19. GGO and bilateral patchy involvement are the most common finding on lung CT scans in children with COVID-19.

Cranial ultrasound and MRI: complementary or not in the diagnostic assessment of children with congenital CMV infection?

Whether or not cranial ultrasound (crUS) and cerebral magnetic resonance imaging (MRI) have both a place in the assessment of children with congenital cytomegalovirus infection (cCMV) remains a topic of discussion between research groups. Literature suggests that MRI is indicated only in children with abnormal crUS.In Flanders, Belgium, combined crUS and MRI was performed on 639 children with cCMV, referred for diagnostic assessment. Cranial US was classified as abnormal in the presence of striatal vasculopathy, calcifications, cysts, cystic germinolysis, and/or ventriculomegaly. MRI findings were classified as abnormal in the presence of gyration disorders, cerebellar abnormalities, ventriculomegaly, cysts, or pathologic white matter lesions.One in five children (93/480) with normal crUS showed abnormal findings on MRI. Of them, 85 (91.4%) were classified as symptomatic. In 37 of those 93 children (39.8%), classification as severely symptomatic was made based on MRI lesions alone. MRI and crUS proved to be complementary in the assessment of CNS involvement in children with cCMV. Long-term studies are needed to evaluate the importance of this finding with respect to outcome and benefit of therapy in this particular subgroup of patients with cCMV infection.Conclusion: Our findings support an enhanced role of MRI in the diagnosis of CNS involvement in children with cCMV infection. The ideal assessment should include both imaging techniques, as the strengths of each test compensate for the other's weaknesses. What is Known: • Congenital CMV infection involves the central nervous system with direct injury to and possible disruption of brain development. • Experts suggest that MRI is indicated only in children with abnormal crUS. What is New: • In almost 20% of our children with a normal cranial ultrasound, abnormalities were detected on MRI. • Our results suggest that performing both MRI and cranial US is important to obtain a complete assessment of central nervous system involvement in children with cCMV.

The relationship between monocyte-to-lymphocyte ratio and the risk of gastrointestinal system involvement in children with IgA vasculitis: A preliminary report.

Immunoglobulin A (IgA) vasculitis is the most common systemic vasculitis of childhood. It can affect the gastrointestinal system (GS) and the renal system. To evaluate the monocyte-to-lymphocyte ratio (MLR) and other hematological markers in predicting GS and renal system complications of IgA vasculitis in children. One hundred and fifteen children with IgA vasculitis and 95 healthy children were included in this study. Demographic characteristics, organ involvement, and laboratory findings, including neutrophil, lymphocyte, monocyte and platelet (Plt) counts, red blood cell volume distribution width (RDW), platelet distribution width (PDW), mean platelet volumes (MPV), monocyte/platelet counts, neutrophil/lymphocyte ratio (NLR), and platelet/lymphocyte ratio (PLR), were evaluated. Among 115 children with IgA vasculitis, 34 (29.5%) cases had GS involvement, and renal involvement was observed in 12 children (10.4%). Neutrophil, monocyte and Plt count and MLR, NLR and PLR values were higher in the IgA vasculitis group than in control groups. Moreover, the neutrophil count and NLR and MLR levels were significantly higher in children with GS involvement than in those without GS involvement. Logistic regression analysis showed MLR was the sole risk factor for GS involvement among these parameters. Furthermore, a cut-off MLR value of 0.245 differentiated children with IgA vasculitis with GS involvement from those without GS involvement (area under the curve (AUC) 0.694, with a sensitivity of 52.9% and specificity of 77.8%). An elevated MLR value could serve as a useful marker in predicting GS involvement in IgA vasculitis in children. Therefore, monitoring the blood MLR value may serve as an important novel indicator to pediatricians regarding the involvement of GS and disease severity of IgA vasculitis.

The Incidence Rate of Central Nervous System (CNS) Involvement in Pediatric Patients Diagnosed with Lymphoblastic Leukemia (ACUTE)

Objectives: The objective of this study is to establish the incidence rate of central nervous system (CNS) involvement in pediatric patients diagnosed with lymphoblastic leukemia (acute). Methodology: A cross-sectional survey was undertaken in the Paediatric Oncology Unit, Sheikh Khalifa Bin Zayyed Hospital, Quetta hospital between 12th January 2021 and August 2022. This research study included 115 patients who fulfilled the eligibility criteria. Demographic profiles were recorded including age, gender and address. Lumbar puncture was done by a single doctor in all these patients at diagnosis to look for CNS involvement. CSF examinations of all patients were done by a single laboratory. Results: The patients had a mean age of 5.9±3.3 years, and the study consisted of 46 (40%) female and 69 (60%) male participants. There were 6 (5.2%) patients who had CNS involvement and 109 (94.8%) had no CNS involvement. In the age range of 1-5, 6-10 and 11-15 years, there were 2 (1.7%) patients who had CNS involvement respectively. In the stratification of CNS involvement with gender, in male patients, there were 2 (1.7%) patients who had CNS involvement and in female patients, there were 4 (3.5%) patients who had CNS involvement. Conclusion: In conclusion, this study reveals that central nervous system involvement in children with acute lymphoblastic leukemia is prevalent upon initial diagnosis. Nevertheless, some disparities have been observed from Western data, such as dissimilarities in age distribution. Keywords: nervous system, Acute lymphoblastic leukemia, white blood cells, total leucocyte counts.

MRI findings of central nervous system involvement in children with haemophagocytic lymphohistiocytosis: correlation with clinical biochemical tests

To investigate the brain magnetic resonance imaging (MRI) features of children with haemophagocytic lymphohistiocytosis (HLH) with central nervous system (CNS) involvement, and to investigate the correlation with clinical biochemical tests. Clinical and MRI data were collected from 118 children with HLH-CNS between January 2012 and June 2019. Patients were grouped according to their MRI findings, and statistical methods were used to test for correlations between the MRI findings and biochemical variables. Patients were divided into three groups, including normal appearance (Group 1, 17/118), diffuse parenchymal volume loss (Group 2, 44/118), and brain parenchyma lesions (Group 3, 57/118) containing three subtypes of brain lesions and HLH-CNS complications. Comparing biochemical values among the three groups revealed a significant difference for all values (p<0.05), except for cell counts in the cerebrospinal fluid (CSF). A pairwise comparison further showed significant inter-group differences for most of the variables. Spearman's rank correlation coefficient also demonstrated that CSF cell counts (r=0.193, p=0.036), CSF microprotein content (r=0.379, p<0.001), serum aspartate aminotransferase (AST; r=0.521, p<0.001), serum lactate dehydrogenase (LDH; r=0.514, p<0.001) and activated partial thromboplastin time (APTT; r=0.326, p<0.001) correlated positively with the MRI groups, while platelet count (PLT; r=-0.633, p<0.001) and plasma fibrinogen (FIB; r=-0.258, p=0.005) correlated negatively. Classification of brain MRI findings of HLH-CNS correlates well with the results of several key biochemical tests. Brain MRI is a promising method to elucidate illness severity and clinical outcomes.

The role of magnetic resonance imaging in the diagnosis of central nervous system involvement in children with acute lymphoblastic leukemia.

Acute lymphoblastic leukemia (ALL) is the most frequent malignancy in childhood. As central nervous system (CNS) involvement requires an intensified CNS-targeted therapy, timely diagnosis is essential. The aim of this retrospective analysis was to evaluate whether cranial magnetic resonance imaging (MRI) examinations findings correlate with cerebrospinal fluid (CSF) analysis on CNS involvement and whether MRI examinations reveal incidental findings with a clinical consequence. All pediatric patients with ALL at our institution between 1998 and 2016 were identified. Patients were divided into two groups: de novo and relapsed ALL. Both groups were analyzed separately for the presence of CNS involvement. Incidental findings were also evaluated. Two hundred fifteen patients with de novo ALL and 31 with relapsed ALL were identified. In the de novo group, no patient was diagnosed CNS positive based on MRI results alone. In relapsed patients, only one patient had a positive MRI with negative CSF results and no neurological symptoms, thus was classified CNS positive solely on the basis of the MRI. In both groups, no patient showed an incidental finding that required therapy. In our study, MRI examinations do not improve the detection of CNS involvement compared with CSF analysis alone. In addition, the analysis of incidental findings does not add value to the performance of an MRI examination performed prior to treatment. Overall, MRI prior to treatment in pediatric patients with ALL is not necessary.

Serum Levels of Interleukin-6 and Tumor Necrosis Factor Alpha in Children With Attention-Deficit Hyperactivity Disorder.

ABSTRACTBackground:Attention-deficit hyperactivity disorder (ADHD) is a common disorder in children, but its etiology and pathogenesis are still unclear.Aims:The aims of this study were to measure the level of serum interleukin-6 (IL-6) and tumor necrosis factor alpha (TNF-α) as markers of immune system involvement in children with ADHD, and to study their correlation with symptoms severity of ADHD.Materials and Methods:The study was conducted on 80 children diagnosed as ADHD based on the criteria adapted from the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition. Eighty healthy children of matched age and sex served as a control group. All children enrolled in the study were subjected to history taking, clinical examination, and psychometric tests. Assay for serum IL-6 and TNF-α for all patients and controls was performed using enzyme-linked immunosorbent assay.Results:The mean serum level of IL-6 was 26.11 ± 11.14 and 6.23 ± 2.52 in children with ADHD and controls, respectively. Children with ADHD showed significantly higher serum IL-6 levels than the control group (P = 0.001). Serum IL-6 showed no significant correlation with the intelligence quotient (IQ) or the Abbreviated Conners’ Rating Scale scores for parents. However, TNF-α showed no significant differences between the two groups and no significant correlation with the IQ or the Abbreviated Conners’ Rating Scale scores for parents.Conclusion:Serum IL-6 levels were significantly higher in children with ADHD compared to controls; however, the IL-6 levels did not correlate with ADHD symptoms severity. Increased IL-6 levels may contribute to the etiology of ADHD.

OAS1 gene polymorphism is associated with central nervous system involvement in children with enterovirus 71 infection

We investigated the association between OAS1 gene polymorphism and susceptibility to central nervous system (CNS) involvement of enterovirus (EV)71 infection. This case-control study was conducted among 180 children with EV71 infection, including 72 with mild infections without any complications and 108 with severe infections and CNS involvement; 201 children undergoing routine physical examination served as the healthy controls. For all the participants, the single nucleotide polymorphisms (SNPs) at OAS1 rs2660 and rs1131454 were analyzed using SNPscan multiple SNP typing methods. No significant differences were found between the case and control groups in genotype or allele distributions of rs2660 and rs1131454. OAS1 rs2660 polymorphism was significantly different between the children with CNS involvement and those with mild EV71 infection, and the genotype AG frequency was higher and the genotype GG frequency was lower in children with CNS involvement. No significant difference was found in the distribution of genotypes or alleles of rs1131454 between the children with CNS involvement and those with mild EV71 infection. OAS1 gene rs2660 and rs1131454 SNPs are not associated with the susceptibility to or CNS involvement of EV71 infection, but OAS1 rs2660 SNPs are significantly correlated with the susceptibility to CNS involvement in EV71 infection. Children carrying OAS1 rs2660 AG genotype are more likely to have CNS involvement after EV71 infection.

Central nervous system involvement in children and adolescents with rhabdomyosarcoma. A report from the AIEOP Soft Tissue Sarcoma Committee.

Rhabdomyosarcoma (RMS) is a highly malignant tumor typically affecting children and adolescents. Central nervous system (CNS) dissemination is rare in RMS patients, but seems to have a particularly negative impact. The aim of this study was to analyze treatment and outcome of patients with RMS and evidence of CNS disease who were registered in the protocols coordinated by the Italian Soft Tissue Sarcoma Committee from March 1979 to December 2016. We analyzed 39 patients with CNS disease. Depending on when their CNS disease was identified, we grouped patients as: Group A, at diagnosis; Group B, progression during treatment; Group C, at first relapse. Six patients were in Group A (2.7% of metastatic RMS patients at diagnosis); 24 were in Group B and 9 in Group C (6.5% of patients with tumor progression/relapse included in the protocols). Only 5 patients (4 in Group A, 1 in Group B) survived the event and are alive in complete remission with a median follow-up of 17.5 years. These 5 patients received systemic chemotherapy and craniospinal radiotherapy, and 2 of them also received intrathecal therapy with topotecan. CNS involvement at diagnosis is a rare and prognostically negative event in RMS patients, but not always fatal when it is found at diagnosis. It is more frequent during or shortly after treatment, and the more dismal prognosis in these cases underscores the need to improve our ability to identify patients at risk of CNS dissemination in order to attempt more effective treatments that can sterilize the meninges.

Use of the Polymerase Chain Reaction as a Complementary Method for the Detection of Central Nervous System Involvement in Children and Adolescents with Acute Lymphoblastic Leukemia.

Cytological analysis of the cerebrospinal fluid (CSF) remains the most widely used method for diagnosing central nervous system (CNS) involvement in acute lymphoblastic leukemia (ALL). This study aimed at evaluating the use of polymerase chain reaction (PCR), in comparison to other methods, for the assessment of the presence of blast cells in the CSF at the time of diagnosis of ALL. This was a prospective, single-centre, study enrolling all patients up to the age of 18 years who were admitted to a university hospital between November 2011 and November 2014 with a diagnosis of ALL and from whom it was possible to draw a sufficient amount of CSF for analysis by conventional cytology (CT), immunophenotyping (IMP), and PCR. A total of 46 CSF samples from 44 ALL pediatric patients were included. CT was performed in all samples, IMP in 44, and PCR in 34. Thirteen (28.2%) samples showed positive results: two by CT, four by IMP, four by PCR, and three by both IMP and PCR. The results of this study showed that PCR should be considered a complementary method for the evaluation of the CSF in ALL patients at diagnosis.

Prognostic analysis of children diagnosed hemophagocytic lymphohistiocytosis with central nervous system involvement in children

Objective To explore the prognostic outcomes of children with hemophagocytic lymphohistiocytosis (HLH) and central nervous system (CNS) involvement. Methods Clinical data of 61 HLH children including CNS symptoms, cranial imaging findings (CT/MRI) and cerebrospinal fluid (CSF) results were collected retrospectively, who were admitted to Hematology Oncology Center of Beijing Children′s Hospital, Capital Medical University from January 2012 to December 2013.The relationship was evaluated between CNS involvement and prognosis by comparing the overall survival (OS) rate. Results In the total 61 cases, median age on diagnosis was 28.1 months, and median follow-up time was 45 months; OS rate was 61.1%(33/54 cases, 7 cases lost follow-up). Thirty-three children (54.1%) were diagnosed as EB virus-HLH, with median age of 48.9 months and OS rate of 69.0% (20/29 cases, 4 cases lost to follow-up), which was higher than other HLHs (52.0%, 13/25 cases), but the difference was not significant(χ2=1.626, P=0.202). There were 24.6% of children with CNS clinical manifestations, and the OS rate was 35.7% (5/14 cases), which was lower than those who had no CNS symptoms (70.0%, 28/40 cases), and the difference was significant (χ2=5.129, P=0.024). There were 61.5% of patients with changed imaging findings, and their OS rate was 54.8% (17/31 cases), which was also lower than those with no changes in CT and/or MRI (87.5%, 14/16 cases), and the difference was significant (P=0.049). While children with abnormal CSF accounted for 19.2% of those who received lumbar puncture, and their OS rate was 40.0% (2/5 cases), which was lower than those with normal CSF (81.0%, 17/21 cases), but the difference was not significant(P=0.101). Any change in CNS symptom, imaging finding or CSF was regarded as CNS involvement.Apart from children who did not have data of CT/MR and/or CSF, the proportion of patients with CNS involvement was 70.4%(38/54 cases), their OS rate was 54.1%(20/37 cases, and 1 case lost follow-up), which was lower than those without CNS impairment (91.7%, 11/12 cases), and the difference was significant (P=0.036). Conclusions Pediatric patients with HLH always have poor prognosis once CNS symptoms or abnormal imaging findings can be founded.CNS involvement is an important risk factor to estimating the patients′ outcome.Cranial MRI and CSF test are necessary for newly diagnosed HLH children, regardless of whether neurological manifestation could be discovered. Key words: Child; Hemophagocytic lymphohistiocytosis; Central nervous system

A 3-year-old boy with Guillain–Barré syndrome and encephalitis associated with Mycoplasma pneumoniae infection

Mycoplasma pneumoniae is a common cause of respiratory tract illness in children. Among the most common extrapulmonary manifestations are disorders of the central nervous system, including meningitis, meningoencephalitis, cerebellitis, polyneuropathy, acute disseminated encephalomyelitis, and Guillain-Barré syndrome. Guillain-Barré syndrome, also known as acute inflammatory demyelinating polyradiculoneuropathy, is an acute-onset, immune-mediated disorder of the peripheral nervous system. The central nervous system is usually intact in patients with Guillain-Barré syndrome. However, there have been some reports of an association of Guillain-Barré syndrome with central nervous system involvement in children. We report a 3-year-old boy with M. pneumoniae infection associated with Guillain-Barré syndrome and encephalitis. Both serum anti-GM1 ganglioside (IgG and IgM) and anti-galactocerebroside IgG antibodies were detected in our patient: the former in the earlier stage of the disease, and the latter in the later stage. We speculate that anti-GM1 ganglioside was associated more with encephalitis, and anti-galactocerebroside antibody was associated more with GBS in our case. Our patient is the youngest report of Guillain-Barré syndrome with central nervous system involvement, and the first report of a pediatric patient with associated M. pneumoniae infection. Such cases are rarely reported, but highlight the need for awareness of the association of the infection with Guillain-Barré syndrome with central nervous system involvement.

Detection of Antiphospholipid Antibody in Children With Henoch-Schönlein Purpura and Central Nervous System Involvement

To explore the mechanisms of central nervous system involvement in children with Henoch-Schönlein purpura, levels of lupus anticoagulant, anticardiolipin antibodies, and anti-β2 glycoprotein I antibodies in serum and cerebrospinal fluid were determined in 46 cases of Henoch-Schönlein purpura with central nervous system involvement. Results indicated that Henoch-Schönlein purpura with central nervous system involvement produced a higher total percentage of antiphospholipid antibodies in serum and cerebrospinal fluid, compared with viral encephalitis control subjects (76.1% vs 10.0% and 71.7% vs 0.0%, respectively; P<0.05). Henoch-Schönlein purpura may be associated with antiphospholipid syndrome or antiphospholipid antibodies, which may account for the neurologic damage in Henoch-Schönlein purpura.

P06.10 Central nervous system involvement in children with autoimmune thyroid diseases

P06.10 Central nervous system involvement in children with autoimmune thyroid diseases

Central Nervous System Involvement in Children with Sarcoma

Objectives: To summarize and analyze the experience in CNS involvement (CNSI) in children with sarcomas treated in the above-mentioned institutions. Patients and Methods: From 1990 to 2001, all medical charts were retrospectively reviewed: 19 sarcoma patients (12 boys and 7 girls) were diagnosed with CNSI (4 osteogenic sarcomas, 11 Ewing sarcomas, 2 rhabdomyosarcomas, 1 alveolar soft part sarcoma and 1 mesenchymal chondrosarcoma). Mean age of all patients at the time of initial diagnosis was 14.9 years (range: 4–24 years), mean age at the time when CNSI was diagnosed was 16.9 years (range: 5.5–27 years). Results: The frequency of CNSI among our patients was 6.17%. The following symptoms and signs (sometimes combined) presented: headache (10 patients), nausea and vomiting (6 patients), seizures (11 patients) and focal neurological signs (9 patients). The mean duration of time elapsed since diagnosis of CNSI till death or last follow-up was 5.2 months (SD: ±5.7 months). Four patients received chemotherapy (CT) alone, 8 CT and radiotherapy (RT), 2 RT alone, 3 supportive treatment only, 1 CT and surgery and 1 surgery alone. Sixteen patients died; there was no significant difference in the duration of survival between those who were treated with RT or surgery (mean ± SD: 6.77 ± 6.56 months) and those who received only CT or supportive treatment (mean ± SD: 2.60 ± 2.94 months) (p = 0.07). Brain disease was the main cause of death in all but 1 patient who died 4 days after autologous bone marrow transplantation from uncontrolled sepsis. In 16 patients, CNSI was part of a metastatic disease. Conclusions: Among children with sarcoma, CNSI is encountered in 6.17% of cases. More effective therapy has to be developed in order to improve their outcome.

Parent pointers☆

We hypothesized that intellectual, neurodevelopmental, and visual-motor tests would be able to characterize the scope and nature of central nervous system involvement in children with congenital central hypoventilation syndrome. Age-appropriate intellectual (Brazelton Neonatal Behavioral Assessment, Bayley Scales of Infant Development, Wechsler Preschool and Primary Scale of Intelligence—Revised and Wechsler Intelligence Scale for Children—Revised), neurodevelopmental (Halsted-Reitan neuropsychologic battery), and visual-motor (Beery visual-motor integration test) tests were given to 17 children with congenital central hypoventilation syndrome (age 0.1 to 14.3 years). We found a broad range in IQ and developmental quotient, from greater than the 85th percentile to less than the 5th percentile, with discrepancies noted between verbal and performance measures. Multiple asymmetries and deficiencies of motor performance, not consistently related to handedness, were also found. Significant eye-hand coordination deficits were uncommon, but general performance was well below average. Full, verbal, and performance IQs and developmental quotients ≥70 and <70 were compared by chi-square analysis with other associated conditions, including hours of ventilatory support, duration of initial hospitalization, growth, pulmonary hypertension, seizures, brain atrophy, central and peripheral hearing deficits, and ophthaimologic abnormalities; no statistically significant associations were found. These results lend support to the hypothesis that congenital central hypoventilation syndrome is a diffuse central nervous system process. However, the effects of transient hypoxia and associated conditions on neurodevelopmental test results cannot be excluded with certainty.

Brainstem auditory evoked potentials and visually evoked potentials in young patients with IDDM.

To investigate whether young IDDM patients develop central nervous dysfunction and to establish a possible relationship with various disease parameters. Thirty-two patients, aged 13.5 +/- 2 years, with disease duration of 6 +/- 2.6 years and age of onset of 7.7 +/- 3.2 years (group 1), and 21 patients with short-term disease, age 9.7 +/- 3.5 years, duration of disease < 2 years and age of onset of 9.4 +/- 3.3 years (group 2) were compared with age- and sex-matched control subjects. Exclusion criteria were clinical signs of neuropathy, retinopathy, nephropathy, or hearing impairment. Neurophysiological studies included auditory and visually evoked potentials (EPs). Patients in group 1 revealed increased P100 latencies of visually EPs (103.4 +/- 4.5 vs. 96.8 +/- 3.7 ms) and interpeak latencies I-V of auditory EPs (4.16 +/- 0.10 vs. 3.99 +/- 0.09 ms) and had abnormal latencies (values outside 2.5 SD) in 37%. However, short-term patients (group 2) had results within normal limits compared with control subjects. In group 1, longer disease duration and younger age at onset correlated with an increase of P100 latency (P < 0.001) and IPL I-V (P < 0.001). Patients with a history of severe hypoglycemic episodes had increased latencies compared with patients without hypoglycemia (P < 0.05). Furthermore, metabolic control during the last 2 years was related to P100 latencies (P < 0.05). EPs noninvasively detect subclinical central nervous system involvement in children and adolescents with IDDM. Most important risk factors are duration of disease and frequency of severe hypoglycemia.